Food or edible material: processes – compositions – and products – Treatment of live animal
Patent
1994-09-12
1996-08-20
Paden, Carolyn
Food or edible material: processes, compositions, and products
Treatment of live animal
426656, A23L 1305
Patent
active
055476872
DESCRIPTION:
BRIEF SUMMARY
BACKGROUND OF THE INVENTION
The present invention relates to a method for removing phenylalanine from proteinaceous compositions, yielding palatable, at least nearly phenylalanine-free protein hydrolysate compositions, a proteinaceous composition thus obtained wherefrom phenylalanine has been removed either totally or at least for the most part, and the use of such a substantially phenylalanine-free proteinaceous composition as a special nutrient product or a component of such, specifically in the diet of patients suffering from phenylketonuria (PKU).
The intake of phenylalanine from food cannot be diminished by selection of the protein source, since phenylalanine is present in all animal and vegetable proteins in an amount of about 4-6%. Thus the PKU patient is well advised to avoid foodstuffs rich in protein, such as egg, fish, meat and cheese. To satisfy the need for other essential amino acids, the patient should use clinical protein products wherefrom phenylalanine has been removed either totally or in part.
Phenylketonuria is a congenital disease that, if untreated, causes serious brain damage and often death within a few weeks from birth. The patient lacks the ability to produce phenylalanine hydroxylase (EC 1. 14.16.1) converting phenylalanine, an amino acid derived from food, to tyrosine. The accumulation of phenylalanine and its pyruvate, lactate and acetate derivatives in the blood and the spinal fluid causes compulsive movements, anaemia, skin roughening and finally brain damage. The disease can also be detected as an increased phenylacetylglutamine content in the patient's urine, wherefrom the disease has derived its name.
In the United States and most of Europe, one newborn in every 11 000-15 000 suffers from phenylketonuria. In Ireland, the disease is more common, one child in every 4 500 being a PKU patient. In Finland, phenylketonuria is very rare, and the proneness of newborns to phenylketonuria is not tested. In countries where PKU occurs, every child is tested within 1 -2 weeks from birth by monitoring the phenylalanine content in the blood. If the amount of phenylalanine in the blood is steadily on the increase, the treatment of the disease should be started three weeks from birth at the latest.
A strictly monitored diet is the only existing practical therapy. When the amount of phenylalanine derived from food can be maintained within certain limits, the phenylalanine content in the patient's blood will remain within allowable limits (20-40 mg/l). Tolerance against phenylalanine increases with age, and it has normally been possible to discontinue the diet at the age of about eight years. According to the most recent results of study, however, the diet should be continued longer, which places even higher requirements on the taste of the special nutrient product.
For the treatment of the disease, phenylalanine-free amino acid mixtures are prepared. Milk for the newborn baby is prepared from such "PKU protein" by adding the other necessary components, such as fat, water, lactose etc., to the protein. Also other ready-prepared PKU products may be produced from PKU protein by replacing the protein used in the production of the foodstuff by a phenylalanine-free amino acid mixture.
Such a commercial preparation is for instance the product PK AID III (manufacturer Scientific Hospital Supplies, England). Products of this kind, comprising substantially only free amino acids, have a bitter taste and may cause osmotic diarrhea. Thus the current recommendation is that the protein nitrogen supplement be derived from an enzymatic hydrolysate comprising oligopeptides, preferably dipeptides and tripeptides, which have been found to be better absorbed in the body than free amino acids (Silk, D. B. A. and Kochane, P. P. in Clinical Nutrition '81, London 1982. Ref. Kochane, P. P. and Silk, D. B. A. in Rombeau, J. L. and Caldwell, M. D., ed., Enteral and tube feeding.
Some methods for the removal of phenylalanine from proteins or proteinaceous compositions are already known. In the oldest of these known m
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Search Report.
Our Response to Search Report.
Response dated Mar. 4, 1994 to the Written Opinion.
Harju Matti
Linko Pekka
Outinen Marko T.
Tossavainen Olli
Paden Carolyn
Valio Oy
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